Impact of clinical history and electrophysiologic characterization of accessory pathways on management strategies to reduce sudden death among children with Wolff-Parkinson-White syndrome

Objectives. This study sought to determine whether the clinical and electrophysiologic criteria developed in adults also identify children with Wolff-Parkinson-White syndrome at risk for sudden death. Background. In adults with Wolff-Parkinson-White syndrome, a shortest RR interval <220 ms during atrial fibrillation is a sensitive marker for sudden death. However, because reliance on the shortest RR interval has a low positive predictive value, the clinical history has assumed a pivotal role in assessing risk. This approach has not been evaluated in children. Methods. We retrospectively evaluated 60 children less than or equal to 18 years old who underwent comprehensive electrophysiologic evaluation between 1979 and 1989 before undergoing operation for Wolff-Parkinson-White syndrome. Clinical and electrophysiologic data were analyzed after patients had been grouped by their clinical presentation: high risk (cardiac arrest), intermediate risk (syncope or atrial fibrillation) or low risk (orthodromic reciprocating tachycardia alone). Results. Ten children had a clinical cardiac arrest (high risk); only one had a prior history of syncope or atrial fibrillation. Compared with the intermediate (n = 19) and low risk groups (n = 31), there were no differences in age ([mean +/- SD] 14.8 +/- 0.6 vs. 14.7 +/- 0.6 vs. 14.5 +/- 1.7 years), duration of symptoms (1.9 +/- 0.5 vs. 4.1 +/- 1.1 vs. 5.2 +/- 0.8 years), incidence of congenital heart disease (30% vs. 26% vs. 32%), presence of multiple pathways (20% vs. 16% vs. 16%) or accessory pathway location. A shortest pre-excited RR interval <220 ms was found in 7 of 7 high risk patients (sensitivity 100%), 14 of 19 intermediate risk patients and 11 of 31 low risk patients (prevalence 35%). Conclusions. Cardiac arrest was the only distinguishing clinical feature between high and low risk groups and the first manifestation in 80% of the children of an accessory pathway that can precipitate a life-threatening arrhythmia. In this series, the largest reported to date of children with Wolff-Parkinson-White syndrome having a cardiac arrest, a shortest pre-excited RR interval <220 ms was more sensitive than clinical history for identifying those at risk for sudden death.